BSPED2009 Poster Presentations (1) (38 abstracts)
Prophylactic Thyroidectomy in Children with Multiple Endocrine Neoplasia Type 2
Clare Morkane 1 , Dimitri Raptis 1 , Caroline Brain 2 , Helen Spoudeas 2 , Mehul Dattani 2 , Peter Hindmarsch 2 , Agostino Piero 2 , Paolo DeCoppi 2 & Tom Kurzawinski 1
1Centre for Endocrine Surgery, University College London Hospital NHS Trust, London, UK; 2Department of Paediatric Endocrinology, Surgery, Great Ormond Street Hospital for Children, London, UK.
Background: The most common cause of death in patients with Multiple Endocrine Neoplasia type 2 is medullary thyroid carcinoma. All patients with MEN2 develop this cancer and Prophylactic Thyroidectomy (PT) is recommended to prevent malignant transformation.
Method: This study reviews our experience of treating children identified as carriers of a RET mutation diagnostic of MEN-2A. Data was collected by reviewing patient notes and hospital electronic databases.
Results: Between 1998 and 2009 15 children (8 boys; 7 girls) were identified by genetic analysis as having MEN 2. The commonest codon with RET mutation was 634Y (n=8), 2 siblings were positive for 891A, 2 further siblings were 790F positive and 1 child had codon 620G mutation. Of these, 13 underwent PT and 3 central lymphadenectomy (2 are awaiting surgery). Median patient age of those undergoing surgery was 7.5 years (range 3.5–15 years) and median hospital stay was 4 days. 10 children had transient hypocalcaemia following surgery and required oral calcium (n=10) and alfacalcidol (n=3). There were no other post-op complications. Histology showed medullary carcinoma in 4 specimens (completely excised) C-cell hyperplasia in 8 cases, and 1 case showed non-specific thyroiditis only. There were no lymph node metastasis and all children but one have undetectable calcitonin levels.
Discussion: This is the first UK case series of children with MEN2 undergoing prophylactic thyroidectomy. We have shown PT to be a rare but safe procedure. We propose to conduct a UK audit of prophylactic thyroidectomy in children with MEN2.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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